Appendiceal neoplasms include a heterogeneous group of epithelial and nonepithelial tumors that exhibit varying malignant potential. This review article summarizes current diagnostic criteria, classification systems, and optimal therapeutic strategies for the five main histopathologic subtypes of appendiceal neoplasms. In particular, the management of epithelial appendiceal neoplasms has evolved. Although their treatment has historically been extrapolated from colon cancer, improved understanding of their unique histopathologic and molecular characteristics and a growing body of published clinical data support a more nuanced approach to their management.